摘要
目的报告1例种痘水疱病样皮肤T细胞淋巴瘤(HV-like CTCL)患者并复习该病临床、组织病理、分子生物学及治疗和预后特点,以提高临床医生对该病的诊治水平。方法分析1例HV-like CTCL患者的临床病理、实验室检查及治疗预后特点,并复习近年国内外相关文献。结果该患者表现为全身丘疹、丘疱疹、坏死、溃疡、结痂和痘疮样瘢痕等多形性皮疹,并伴有浅表淋巴结肿大。组织病理示真皮全层及脂肪小叶间隔密集的淋巴细胞浸润,细胞有异型性;免疫组化示肿瘤细胞表达CD2、CD3、CD8、TIAI、GramB,CD20,CD30,CD56阴性。皮损基因重排示TCR-δ(+)。原位杂交检测Epstein-Barr(EB)病毒(+)。患者经干扰素治疗病情缓解。结论 HV-like CTCL临床表现特殊,主要依靠组织病理及分子生物学手段确诊。目前该病没有统一的治疗方案,其预后与患病年龄、发病诱因(如血液EB病毒滴度、亚型和基因变异)及治疗等多种因素相关。
Objective To report a case of hydroa vaccinifoime-like T cell lymphoma （HV-like CTCL） and review the futures of its clinicohistopathology, molecular biology, therapy and prognosis. Methods The clinical, histopathologic futures, laboratory tests, therapy and prognosis were summarized. Literatures about HV-like CTCL were reviewed. Results The patient presented with polymorphous lesions including papules, papulovesicle, necrosis, ulceration, crusting and smallpox- like scar. Histopathological examination exhibited diffuse and dense infiltrate of atypical lymphcytes in the dermis and subcutaneous. The atypical cells were positive stained with CD2, CI）3, CD8, TIAI and GramB; CD20, CD30 and CD56 were negative. Monoclonal gene rearrangement of TCR-~ was found. The EB virus was detected by insitu hybridization. The patient responded well to interferon treatment. Conclusion The diagnosis of HV-like CTCL could be confirmed on the special clinical manifestations, the atypical lymphocyte infiltrate and the molecular biology study. No uniform treatment program was accepted. The prognosis was related to the age of onset, the treatment and associated incentives factors such as the blood concentrations, virus subtypes and genetic variation of EB virus.
出处
《中国中西医结合皮肤性病学杂志》
CAS
2014年第1期20-23,共4页
Chinese Journal of Dermatovenereology of Integrated Traditional and Western Medicine
